Kim Tara S, Cheng Kwan, Jaiswal Radhika, Gautam-Goyal Pranisha, Myers Alyson K
Department of Internal Medicine, Division of Endocrinology, Lenox Hill Hospital, New York, New York.
David and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, New York.
J Endocr Soc. 2020 Oct 30;5(1):bvaa156. doi: 10.1210/jendso/bvaa156. eCollection 2021 Jan 1.
Adrenal incidentalomas, masses noted on imaging performed for other purposes, are common, with 10% to 15% presenting as bilateral adrenal masses. These cases can be challenging as the differential diagnosis is broad, including metastatic disease, primary adrenal lymphoma (PAL), or infection, and often requiring a biopsy if initial biochemical workup is unrevealing. We present here a relevant case description, laboratory and radiologic imaging studies, and discussion of literature. A 62-year-old Korean woman presented with altered mental status and fevers. She was found to have bilateral adrenal incidentalomas and retained acupuncture needles. Adrenal workup did not show biochemical evidence of hormonal excess. Infectious workup was unrevealing, as was a metal/toxin workup due to retained acupuncture needles. Fevers and episodes of hypotension persisted which prevented the patient from obtaining an adrenal biopsy. Bone marrow biopsy was obtained for pancytopenia and revealed B-cell lymphoma with large cell morphology and few histiocytes with hemophagocytosis, raising concern for lymphoma-induced hemophagocytic lymphohistiocytosis (HLH). PAL associated with HLH was highly suspected in our patient, given the large (7 cm) bilateral adrenal masses and bone marrow biopsy findings of lymphoma. The patient was treated for diffuse large B-cell lymphoma, with clinical improvement. PAL is a rare but aggressive lymphoma with few reported cases. It should be considered in the differential for both unilateral and bilateral adrenal masses. An early diagnosis is crucial as the main treatment is chemotherapy rather than surgery and it confers a significant survival benefit.
肾上腺偶发瘤是在因其他目的进行的影像学检查中发现的肿块，很常见，10%至15%表现为双侧肾上腺肿块。这些病例具有挑战性，因为鉴别诊断范围广泛，包括转移性疾病、原发性肾上腺淋巴瘤（PAL）或感染，如果初始生化检查无异常发现，通常需要进行活检。我们在此呈现一个相关病例描述、实验室及放射影像学研究，并讨论相关文献。一名62岁的韩国女性因精神状态改变和发热就诊。她被发现有双侧肾上腺偶发瘤并留存有针灸针。肾上腺检查未显示激素过量的生化证据。感染性检查无异常发现，因留存针灸针进行的金属/毒素检查也无异常。发热和低血压发作持续存在，这使得患者无法进行肾上腺活检。因全血细胞减少进行了骨髓活检，结果显示为具有大细胞形态的B细胞淋巴瘤，仅有少数组织细胞伴噬血细胞现象，这引发了对淋巴瘤诱导的噬血细胞性淋巴组织细胞增生症（HLH）的担忧。鉴于双侧肾上腺肿块较大（7厘米）且骨髓活检发现淋巴瘤，我们高度怀疑患者患有与HLH相关的PAL。患者接受了弥漫性大B细胞淋巴瘤的治疗，临床症状有所改善。PAL是一种罕见但侵袭性强的淋巴瘤，报道病例较少。对于单侧和双侧肾上腺肿块的鉴别诊断均应考虑到它。早期诊断至关重要，因为主要治疗方法是化疗而非手术，且能带来显著的生存获益。
